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COM - Repro > Development Of The Genital System - 2/19 Cole > Flashcards

Development Of The Genital System - 2/19 Cole Flashcards

1
Q

When does phenotypic sexual differentiation begin?

Complete?

A

Week 7

Week 20

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2
Q

What do primordial germ cells originate from?

A

Endoderm of yolk sac

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3
Q

What guides the migration that is expressed on PGCs?

Failure of PGCs to reach the genital ridge leads to what?

A

C-kit receptor and stem-cell factor (c-kit ligand)

Gonadal dysgenesis

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4
Q

What do Sertoli cells secrete?

What does this do?

A

MIS/MIF/Anti-mullerian hormone

Regression of Paramesonephric (Mullerian) Ducts

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5
Q

Where are leydig cells located?

What do they secrete?

A

Interstitial cells

Testosterone

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6
Q

What is critically important to ensure the formation of Sertoli cell formation and differentiation?

A

SOX9 and SF1

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7
Q

What is characterized by short limbed dwarfism, bowing of long bones and skeletal features like hypoplastic lungs, malformations of cervical spine, heart and kidneys?

Due to what?

A

Campomelic dysplasia

SOX9

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8
Q

What is DHT mainly responsible for in males?

A

Masculinization of external genitalia and prostate

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9
Q

What does testosterone do after released from leydig cells?

A

Masculinization of reproductive system (Wolfian duct)

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10
Q

Which kind of 5a-reductase is made by reproductive tract?

Where?

A

Type II

Epididymis, seminal vesicle, prostate

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11
Q

When do the primary (medullary) cords degenerate by in females?

A

10th week

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12
Q

In females the mesonephric ducts degenerate except for what?

Where are they found?

A

Epoophron and paroophoron

Mesentery of ovary

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13
Q

What are portions of the mesonephric duct left in the female called?

A

Gartner’s duct

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14
Q

What up regulates DAX1 in the female?

What does DAX1 do?

A

WTN4

Blocks SOX9 and AMH

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15
Q

When do the Paramesonephric (Mullerian) ducts develop?

Where?

A

6th week

Lateral to mesonephros

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16
Q

The paired portions of the Paramesonephric ducts become what?

A

Uterine tubes

17
Q

What does the uterovaginal primordium become?

A

Epithelium and glands of the uterus, epithelium of upper 1/3 of vagina, muscular wall of vagina

EEM

18
Q

Where does the inferior portion of the vagina form from?

Tissue from where?

A

Posterior wall of Urogenital sinus

Sinovaginal bulbs

19
Q

When can a 46 XY male have persistent Paramesonephric ducts?

A

Low AMH or abnormal response to AMH

20
Q

What is MRKH?

A

Failure of the Paramesonephric ducts to develop

Missing uterine tubes, uterus and variable malformation of the upper portion of the vagina

21
Q

Imperforate hymen can cause what?

A

Primary amenorrhea (absence of menses)

Hematosalpinx -> endometriosis and adhesion formation

Hemoperitoneum

22
Q

What does the genital tubercle form in the male?

Urethral folds?

Labioscrotal swellings?

A

Phallus

Penile urethra

Scrotum

23
Q

In the female, what does the genital tubercle become?

Urethral folds?

Labioscrotal swellings?

A

Clitoris

Labia minora

Labia majora

24
Q

What is cryptorchidism?

A

Undescended testes

Usually descend in 1st year, associated with increased testicular cancer

25
Q

What is hypospadias?

What causes it?

A

Urethra opens onto the ventral aspect of penis

Inadequate androgens or inadequate receptor sites for DHT

26
Q

What is epispadias?

What does it occur with?

A

Urethral meatus opens on dorsal side of penis

Bladder exstrophy

27
Q

Genotype of Klinefelter syndrome?

Characteristics?

A

47 XXY

Small testes, low levels of testosterone, elongate limbs, poor development of secondary sexual characteristics

28
Q

Genotype of Turner’s syndrome?

Characteristics?

A

45X or Mosiac 46XX

Short, no adolescent growth, broad chest, CHD

Secondary sex characteristics do not develop

29
Q

What is pseudo intersexuality?

Genotype?

A

External genitalia of one sex accompanies gonads of the other sex

46XX or 46XY

30
Q

What can cause 46XX DSD?

Exposure after 12th week leads to what?

A

Prenatal exposure to androgens

Clitoris hypertrophy

31
Q

Genotype of AIS?

Cause?

Characteristics?

A

46 XY DSD

X-linked disorder where receptors unresponsive to androgens

External genitalia are feminine, undescended testes

32
Q

5a-reducatase deficiency (5-ARD) is failure of what?

Result?

Genotype

A

Inability to convert testosterone to DHT

Born with ambiguous genitalia, normal mesonephric duct derivatives, “first women, then man)

46XY DSD

33
Q

When can M/F external genitalia be recognized?

A

Week 12

COM - Repro (15 decks)

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